Abstract
IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.
Highlights
Type 1 autoimmune pancreatitis (AIP) and immunoglobulin G4- (IgG4-) associated sclerosing cholangitis (IgG4-SC) are pancreatic and biliary manifestations of IgG4-related disease, respectively
IgG4-related disease has emerged as a novel clinical entity characterized by multiorgan infiltration of IgG4-positive cells, storiform fibrosis, and elevated serum IgG4 levels
The patient was started on 40 mg of prednisone and continued for 4 weeks and gradually tapered. His symptoms abated and a follow-up Magnetic resonance imaging (MRI) performed demonstrated complete resolution of the pancreatic mass and biliary strictures (Figures 1(b) and 1(d)). With this clinical and radiologic response, the patient was diagnosed with IgG4-related SC and the symptoms have not recurred after 3 years of follow-up
Summary
Type 1 autoimmune pancreatitis (AIP) and immunoglobulin G4- (IgG4-) associated sclerosing cholangitis (IgG4-SC) are pancreatic and biliary manifestations of IgG4-related disease, respectively. IgG4-related disease has emerged as a novel clinical entity characterized by multiorgan infiltration of IgG4-positive cells, storiform fibrosis, and elevated serum IgG4 levels. Often the differential diagnosis for AIP/IgG4-SC includes malignant conditions such as pancreatic cancer and cholangiocarcinoma, placing patients at risk for unnecessary surgery for a more benign condition. Current clinical diagnostic criteria for type 1 AIP/IgG4-SC involve elevated serum IgG4 concentrations (>135 mg/dL). In patients with normal serum IgG4 levels, a thorough workup including pancreatic and biliary imaging, histology, and identification of extrapancreatic manifestations aids in accurate diagnosis. IgG4 negative type 1 AIP/IgG4 SC that responded to a trial of steroid therapy, allowing for the avoidance of surgery
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